Gastroesophageal reflux following repair of esophageal atresia and tracheoesophageal fistula.

OBJECTIVE: This study represents the experience of a tertiary care center in the Kingdom of Saudi Arabia (KSA) on the long-term effect on the lungs of esophageal atresia (EA) and tracheoesophageal fistula repair (TEF), and to emphasize the magnitude of gastroesophageal reflux (GER) post-EA or post-TEF repair. METHODS: A retrospective review of all patients referred to the pulmonary clinic with EA/TEF or re-operative evaluations from the period 1993-2004 at King Faisal Specialist Hospital and Research Centre, Riyadh, KSA. RESULTS: Forty-one patients with confirmed EA/TEF (26 males and 15 females) were included in the study. Congenital anomalies were associated in 28 (68%). Gastroesophageal reflux developed in 39 (95%) of patients, 24 (59%) needed Nissen fundoplication. Esophageal stricture that required more than 3 dilations developed in 16 (46%) patients, esophageal dysmotility in 37 (90%) and hiatal hernia in 11 (27%). Pulmonary complications developed in >70% of the patients including persistent atelectasis, chronic aspiration pneumonia, asthma and chronic lung disease that required oxygen for more than one month. Tracheomalacia in 12 (29%) and bronchiectasis in 7 (17%). Eighty-eight percent of patients who were able to do pulmonary function test showed abnormal values of moderate obstructive and restrictive lung disease. CONCLUSION: Pulmonary complications cause significant and prolonged morbidities post EA/TEF repair. Gastroesophageal reflux is a common complication after EA/TEF repair and causes significant morbidity that needs a prolonged follow up. Patients with GER may need Nissen fundal plication to improve respiratory problems.

Pediatric scoliosis and the lung.

OBJECTIVE: To determine the pattern of scoliosis in the pediatric age group and to determine the course of the disease and the result of surgical intervention. METHODS: A retrospective review of all charts of patients referred to the Pulmonary Clinic of the King Faisal Specialist Hospital, Riyadh, Kingdom of Saudi Arabia (KSA), for scoliosis and preoperative evaluation from the period November 1992 to October 2001. Demographic and clinical data, preoperative and postoperative pulmonary function test (PFT) and effect of surgery on the lung were presented. RESULTS: A total of 66 patients with scoliosis were followed. The age at diagnosis was 8.5 +/- 4.4 years. Thirty-four (52%) males and 32 (48%) females. Fifty-seven (86%) were alive and 9 (14%) died. A period of follow up was 5.3 +/- 3.7 years. Idiopathic scoliosis was found in 20 patients (30%), congenital anomalies associated with scoliosis constituted 35 (53%) of the total scoliosis referral and secondary type due to trauma or malignancy formed 11 (17%). Mean Cobb s angle of 70 +/- 30 degrees. The most common curvatures were: thoracic 31 (47%), lumbar 12 (18%) and thoracolumbar 30 (45%). Thirteen patients (20%) had 2 or 3 types of curvatures simultaneously. Baseline PFT showed moderate restrictive lung disease in approximately two thirds of the patient population. Scoliosis surgery was carried out in 34 (52%) of the patients. Posterior approach was more commonly used in 30 (88%) of the patients. Pneumonia and atelectasis were the most common complication encountered in 15 (44%) of the patients. Recurrence of scoliosis after operation recurred in 12 (35%) of the population. Comparisons between both groups have shown that patients with congenital anomalies and scoliosis were diagnosed earlier, had significant postoperative complications and higher mortality compared to those with idiopathic scoliosis (p<0.05). Changes in PFT for patients who underwent surgery for scoliosis with Cobb s angle >40 degrees showed less deterioration compared to those with angle <40 degrees (p=0.004), due to progressive deterioration without surgery. CONCLUSION: Scoliosis is a common problem in KSA. Patients need to be referred early for proper intervention before it becomes a severe magnitude and difficult to be fixed and to prevent permanent complications. Patients with congenital anomalies and scoliosis had poor prognosis compared to those with idiopathic scoliosis.

Sickle cell disease and cystic fibrosis.

Simultaneous occurrence of both cystic fibrosis and sickle cell disease in the same patient is a rare phenomenon and has occurred only in 3 reports in the world literature. This phenomenon has never been described before in Arab population. In this report, we describe the first reported case of both diseases in one patient in the Arab population, and discuss its complications and review the literature in this aspect.

Cystic fibrosis: presentation with other diseases, the experience in Saudi Arabia.

Simultaneous occurrence of Cystic fibrosis and other inherited diseases or congenital anomalies has been rare. This association has never been described before in the Arab population. In this report we describe the first report on cystic fibrosis in association with other diseases in the same patient such as sickle cell disease, Insulin dependant Diabetes mellitus, congenital adrenal hyperplasia, cardiac anomalies in twins and Ehler's Danlos syndrome. We also evaluate their effects on CF patients and review the literature in this aspect.

Diabetes mellitus and cystic fibrosis in 2 Saudi siblings.

Diabetes mellitus and cystic fibrosis (CF) have been reported before in the literature, but they have never been reported in the same patient in the Middle East. We present the first reported case of insulin dependent diabetes mellitus (IDDM) and CF in 2 siblings of the same family. Both siblings were diagnosed early in life with IDDM, and their diabetes was well controlled on insulin. Cystic fibrosis was diagnosed in the first case one year after IDDM was diagnosed due to history of chronic cough and in the 2nd case by family screening. Both had severe failure to thrive, recurrent chest infections and gastro-esophageal reflux. With treatment both showed clinical improvement, but continued to have moderate lung disease radiologically and by pulmonary function test.